The kidney in plasma cell dyscrasias

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Essentials of Diagnosis

Jimena Cabrera. Esther Gonzalez. Amado Andres. Cite Citation. Permissions Icon Permissions. All rights reserved.

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Issue Section:. Download all figures. Comments 0. Add comment Close comment form modal. I agree to the terms and conditions. You must accept the terms and conditions. Add comment Cancel. Submit a comment. Comment title. You have entered an invalid code. Serious and potentially life-threatening paraneoplastic complications can occur in plasma cell dyscrasias regardless of tumor cell burden, myeloma protein levels, or the presence of other criteria suggesting the dyscrasia has entered a malignant phase.

Many of these complications are caused by the tissue-destructive effects of the myeloma proteins, are predictive of a rapidly progressive disease, and require chemotherapeutic or other treatments directed at lowering the burden of the myeloma protein-producing cells. The serious paraneoplasitic diseases that complicate the plasma cell dyscrasias and may require such treatments include the following. Amyloidosis is a general term for a protein misfolding syndrome that involves the deposition of a low molecular weight beta-pleated sheet -containing protein in extracellular tissues.

These proteins normally circulate in the blood but may undergo conformational changes that cause them to auto-aggregate along their beta-pleated sheets to become insoluble and form fibril deposits in and outside of the circulation. These deposits disrupt tissue architecture and, in the case of light chains, directly injure cells, thereby causing potentially cataclysmic organ failures. There are 31 types of circulating proteins that can become misfolded and lead to distinctly different types of amyloidosis; among these, myeloma proteins, particularly free light chains, are the predominant cause of the disease.

The deposits in a third type, AHL amyloidosis , consists of both free light chains and free heavy chains. AHL amyloidosis is here, as in some recent reports, grouped with AH amyloidosis. AL amyloidosis can occur at any stage in the plasma cell dyscrasia spectrum. They may also exhibit evidence of liver involvement liver failure, increases in circulating liver enzymes, bleeding due to factor X deficiency , gastrointestinal track deficiencies malabsorption , and amyloid deposition in surface tissues macroglossia , shoulder pad masses , cutaneous nodules.

Arthritis in multiple joints, often manifested before diagnosis, is also a common feature of AL amyloidosis and has led to initial misdiagnoses of rheumatoid arthritis. Treatment commonly improves this poor survival. In a Mayo Clinic study, for example, AL amyloidosis assigned stage 1, 2, 3, or 4 based on the presence of 0, 1, 2, or 3 prognostic signs high blood levels of [cardiac troponin T] , blood levels of a marker for congestive heart failure viz.

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AH and AHL amyloidosis are extremely rare forms of systemic amyloidosis in which the amyloid deposit is a free heavy chain AH amyloidosis or a free heavy chain plus free light chain AHL amyloidosis. The hematological response to chemotherapy of the AH and AHL renal amyloidosis patients was comparable to those with renal AL amyloidosis.

POEMS syndrome also known as Crow—Fukase syndrome, Takatsuki disease, or PEP syndrome is a rare and complex medical syndrome that involves a combination of syndrome-defining signs and symptoms due to the dysfunction of multiple organs. Rare cases of POEMS have been associated with polyclonal rather than clonal plasma cells; these cases are not plasma cell dyscrasias but rather appear to be caused by the over-activity of non-malignant immune cell responses in chronic infections or autoimmune diseases.

Deletion of chromosome 13 and chromosomal translocations but not increases in chromosome number have also been reported to occur in POEMS patients. Patients with 1 or 2 isolated plasmacytomas have been successfully treated with targeted radiotherapy to obtain relief of symptoms and sometimes complete remission of disease. Isolated plasmactyomas may regress spontaineously. They are made and secreted into the blood as a result of underlying pathological conditions viz. Rarely, cryoglobulinemia i. Non-essential cryoglobulonemia is classified into three types.

Only types I and II are defined as plasma cell dyscrasias. Patients suffering type 1 cryoglobulinemia present with symptoms due to cold temperature-induce blood hyperviscosity and consequential interruptions of blood flow, e. Rarely, patients may present with catastrophic decreases in blood flow to vital tissues and require emergency treatment. Patients with an overt malignancy are treated with the chemotherapy regimens used for Waldenstroms macroglobulinemia or multiply myeloma; patients with MGUS precursors to these diseases appear less reaponsive to these chemotherapeutic regimens.

These patients as well as patients with overt malignancy may be treated with rituximab kills normal and malignant B cells that bear the CD20 antigen or the proteasome inhibitor , Bortezomib. Patients suffering type II or type III cryoglobulinemia present with many of the symptoms of type I disease plus those of inflammatory vasculitis.

Their treatments are tailored to the underlying infectious, autoimmune, or malignant disease. Type II patients associated with a monoclonal antibody and clonal plasma cells or other types of clonal B cells, are typically treated with regimens used for Walsdenstorms macroglobulonemia or multiple myeloma.

The Kidney in Plasma Cell Dyscrasias

In the malignant stage of plasma cell dyscrasias, a clearly excessive tumor cell burden causes symptoms and findings predictive of rapid, life-threatening progression of disease. These dyscrasias fall into several distinct categories. Solitary plasmacytoma is an early stage malignancy with a clinical course that lies between MGUS and multiple myeloma in the spectrum of plasma cell dyscrasias. Overall median survival was 8. While its presentations and findings are similar to solitary plasmacytoma, solitary plasmacytoma with minimal bone marrow involvement is more likely to progress, i.

While a pre-malignant phase is likely, most new cases of non-secretory multiple myeloma are brought to attention not because of incidental M protein detection which by definition is absent but because of patient symptoms indicative of malignancy possibly of plasma cell origin. These patients typically also show one or more CRAB signs and lack evidence of a myeloma protein as measured by protein electrophoresis and immunofixation. As a group, these patients response to therapy, time to disease recurrence, and overall survival were similar to typical myeloma patients.

However, in a subset of patients diagnosed after and therefore treated with more effective therapy that included autologous stem-cell transplantation , prognosis was significantly better in non-secretory multiple myeloma patients median survival 8.

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In addition, non-secretory patients exhibited a better prognosis than light chain-secretory patients. Multiple myeloma occurring concurrently with chronic Lymphocytic Leukemia or its pre-malignant precursor, monoclonal B-cell lymphocytosis , is an extremely rare condition in which patients evidence findings of the plasma cell dyscrasia plus either one of the cited clonal lymphocytic diseases.


Signs and symptoms of chronic lymphocytic leukemia commonly precede those of multiple myeloma, sometimes by years. Some patients who lack appreciable symptoms have been followed with no specific treatment of their disease. There is no requirement for symptomatic disease, a particular level of IgM protein, or presence of extramedullary i. Light chain multiple myeloma is diagnosed in patients who have: a the criteria for diagnosis of multiple myeloma except having a serum free light chain ratio outside the normal range of 0.

Patients are treated similarly to patients suffering the counterparts those with multiple myeloma except that the focus is treating or preventing kidney damage using chemotherapy to reduce production of the monoclonal light chain and thereby stopping, reversing, or preventing kidney injury. Plasma cell leukemia is a form of multiple myeloma in which significant numbers of typically immature appearing plasma cells, i. In these plasma cell dyscrasias, the presence of even very small numbers of circulating plasma cells is a poor prognostic indicator. In plasma cell leukemia, however, circulating plasma cells reach far higher numbers and at these circulating levels are associated with exceptionally poor survival rates.

More recently, the Group has suggested that values of 0. There are two forms of plasma cell leukemia: Primary plasma cell leukemia in which patients without a history of multiple myeloma present with diagnostically high levels of circulating plasma cells and Secondary plasma cell leukemia in which patients with multiple myeloma suffer their dyscrasia's progression by the expansion of large numbers of their malignant plasma cells into the circulation and distant tissues.

Historically, primary plasma cell leukemia was more common than the secondary form but with the increased survival of multiple myeloma patients due to new treatment regiments, more cases of secondary plasma cell leukemia are occurring; currently, the two forms occur in approximately equal numbers. They are less likely than multiple myeloma patients to have lytic bone lesions.

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant.

Acute Kidney Injury and Plasma Cell Dyscrasia - Renal and Urology News

Diagnosis of monoclonal gammopathy of renal significance. Kidney Int. Serum free light chain measurement aids the diagnosis of myeloma in patients with severe renal failure. BMC Nephrol. Bridging the Divide: An onco-nephrologic approach to the monoclonal gammopathies of renal significance. Clin J Am Soc Nephrol. Am J Hematol. IgD heavy-chain deposition disease : detection by laser microdissection and mass spectrometry.

J Am Soc Nephrol. Laser microdissection and mass spectrometry-based proteomics aids the diagnosis and typing of renal amyloidosis. Monoclonal gammopathy of renal significance: Diagnostic workup.

The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias
The kidney in plasma cell dyscrasias The kidney in plasma cell dyscrasias

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